{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T09:22:58Z","timestamp":1792142578435,"version":"4.4.0"},"reference-count":83,"publisher":"S. Karger AG","issue":"5","license":[{"start":{"date-parts":[[2007,1,1]],"date-time":"2007-01-01T00:00:00Z","timestamp":1167609600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2007,1,1]],"date-time":"2007-01-01T00:00:00Z","timestamp":1167609600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Respiration"],"published-print":{"date-parts":[[2007]]},"abstract":"<jats:p>\u03b11-antitrypsin deficiency is a genetic disorder which contributes to the development of chronic obstructive pulmonary disease, bronchiectasis, liver cirrhosis and panniculitis. The discovery of \u03b11-antitrypsin and its function as an antiprotease led to the protease-antiprotease hypothesis, which goes some way to explaining the pathogenesis of emphysema. This article will review the clinical features of \u03b11-antitrypsin deficiency, the genetic mutations known to cause it, and how they do so at a molecular level. Specific treatments for the disorder based on this knowledge will be reviewed, including \u03b11-antitrypsin replacement, gene therapy and possible future therapies, such as those based on stem cells.<\/jats:p>","DOI":"10.1159\/000105536","type":"journal-article","created":{"date-parts":[[2007,7,31]],"date-time":"2007-07-31T10:06:17Z","timestamp":1185876377000},"page":"481-492","source":"Crossref","is-referenced-by-count":61,"title":["Alpha One Antitrypsin Deficiency: From Gene to Treatment"],"prefix":"10.1159","volume":"74","author":[{"given":"Alice M.","family":"Wood","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert 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